GNAS pathogenic variants have been previously found in various systems and has been reported to be associated with many extra-skeletal diseases such as thyroid hyperfunction, hormone-secreting pituitary tumors, pancreatic cancer, breast cancer and colorectal cancer [6–9, 17, 18, 32, 33].Furthermore, GNAS pathogenic variant is also detected in an endothelial meningioma with multiple recurrences recently [34]. Here, GNAS is linked to pancreatic neoplasm.